Congenital portosystemic shunt characterized by variable absence of intrahepatic portal vein (PV)
Type 1: Absence of intrahepatic PV with extrahepatic portosystemic shunt
Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
Type 2: Intact but hypoplastic intrahepatic PV with extrahepatic portosystemic shunt
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Baiges A et al: Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology. 71(2):658-69, 2020
Bueno J et al: Radiological and surgical differences between congenital end-to-side (Abernethy malformation) and side-to-side portocaval shunts. J Pediatr Surg. 55(9):1897-902, 2020
DiPaola F et al: Congenital portosystemic shunts in children: associations, complications, and outcomes. Dig Dis Sci. 65(4):1239-51, 2020
Rajeswaran S et al: Abernethy malformations: evaluation and management of congenital portosystemic shunts. J Vasc Interv Radiol. 31(5):788-94, 2020
Sokollik C et al: Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 56(6):675-81, 2013
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KEY FACTS
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TERMINOLOGY
Synonyms
Congenital extrahepatic portosystemic shunt
Definitions
Congenital portosystemic shunt characterized by variable absence of intrahepatic portal vein (PV)
Type 1: Absence of intrahepatic PV with extrahepatic portosystemic shunt
Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
Type 2: Intact but hypoplastic intrahepatic PV with extrahepatic portosystemic shunt
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Baiges A et al: Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology. 71(2):658-69, 2020
Bueno J et al: Radiological and surgical differences between congenital end-to-side (Abernethy malformation) and side-to-side portocaval shunts. J Pediatr Surg. 55(9):1897-902, 2020
DiPaola F et al: Congenital portosystemic shunts in children: associations, complications, and outcomes. Dig Dis Sci. 65(4):1239-51, 2020
Rajeswaran S et al: Abernethy malformations: evaluation and management of congenital portosystemic shunts. J Vasc Interv Radiol. 31(5):788-94, 2020
Sokollik C et al: Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 56(6):675-81, 2013
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